At this meeting, group members heard from a panel of individuals representing the myositis health team from both personal and professional perspectives. A myositis patient participated along with the four members listed above; she was diagnosed with myositis in May of 2000, and has been in remission and has been taken off all medications for at least 5 months. Following the panel presentations, Ms. Horton turned to the audience for a question-and-answer session. Participants touched upon questions having to do with myositis symptoms, medication, treatment, remission, and the future of myositis care.

Does inflammation destroy the muscles?

In response to the question, Dr. Kagen said that inflammation is a process that has evolved for a reason. Inflammation is a defense mechanism that is available for everyone. It represents the enforcement of the rules that govern our bodies. However, you tend to look at inflammation as kind of a bad thing when you have myositis because the inflammation doesn’t stop -- it just keeps going and going, and normal tissue can be lost in the process. Inflammation is the defense mechanism that we rely on to protect us, but when it acts up and we take anti-inflammatory medications, we may be subject to infection.

It was mentioned that myositis patients should avoid alcohol. Why?

Dr. Kagen shared that consuming alcohol can produce muscle changes and raise the creatine phosphokinase (CPK) level, which can prevent a proper evaluation of a person’s progress. When patients come in with elevated CPK levels, a doctor may think that a particular treatment isn’t working, but then the patient mentions that they had a glass of wine or a beer the night before. Therefore, the activity of an individual’s disease can be determined more accurately if a patient avoids alcohol.

What are the effects of long term use of steroids like prednisone?

Dr. Kagen discussed how steroids often produce weakness through atrophy of muscle fiber. Weakness often occurs as muscle fibers are destroyed from inflammation or when muscle fibers are less efficient in the arena of inflammation. If the efficiency of muscle function is causing the weakness, then strength can return when the steroids are stopped. We often can improve but not necessarily recover to the same strength maintained prior to the use of steroids. Also, the ability to regenerate muscle declines with age.

What kind of experience did the patient participant have with her medications?

She said that she experienced several side effects. One made her hair curly, and another prevented her from sleeping at night. She also experienced headaches. As the dosages of medications were raised and lowered to a level her body could handle, she did not have as many side effects. She also mentioned how difficult it was for her to give herself injections twice a week. In addition, she had a home care nurse providing infusion in her home, and by the time she finished infusion, the nurse could hardly find a vein. She honestly shared that it never got easier for her to go through constant medical treatments. She said it was scary to have a needle coming at her. She even started to leave the needle in her arm and sleep with it for four days in a row.

Although she did experience all of these side effects with her medications, she noted that her health was the most important thing and that she was very diligent about following her doctor’s guidance. Eventually, she felt herself getting better.

What makes a good candidate for infusion?

Dr. Kagen shared that most patients prefer to start with oral medications, and many of them work. However, for patients considering infusion, there are a number of factors to consider. Infusion takes time. It requires a patient to devote a number of hours per day for several days in a row. Also, infusions are extremely expensive and would be almost impossible to afford if an insurance company did not cover the cost. The government just this year allowed for the possibility to reimburse patients receiving Medicare for all three types of myositis. Another reason why some people are good candidates and others are not is that some people may experience unusual reactions, such as headaches or problems related to the cardiovascular system. Other patients do not have good venous access, and not all people have the ability to get prodded and stuck over and over. Unfortunately, there are even times when IVIG is difficult to get when there is a shortage. Infusions can be valuable to patients, but they are not a cure and can have side effects.

Are there treatments that can help people with IBM?

Dr. Kagen shared that inclusion body myositis (IBM) more often occurs in patients that are older. The overall success rate with treatments for IBM patients is not very good, and many doctors feel that patients shouldn’t be subjected to the risks of medications with little hope of benefit. Other doctors may feel that it’s worthwhile to give it a try. Some studies may suggest that particular IBM patients do not improve, but the rate of decline in their health may be affected. Some patients are not doing better, but they are maintaining better and not losing their fight the way they were losing before.

Is it possible that a patient is receiving too high of a dosage of IVIG?

One group member mentioned that she had two infusions instead of four last month and that her physical therapist noticed that her movements are much better and her walking has improved. Ms. Leff pointed out that medications are weight-based and are adjusted as a person gains or loses weight, so she probably wasn’t taking too much to begin with. The member said her improvement could be the result of being saved from two days of waking up at 5 am and returning home at 6 or 7 pm at night. Dr. Kagen recognized that infusion could be very difficult for many people because the procedure is labor intensive; not everyone can tolerate an 8-hour-a-day treatment. Just getting to the infusion unit can be exhausting. Also, Ms. Leff pointed out that the medication itself has a side effect of fatigue. Sometimes, a patient may break up their appointments by coming in on a Thursday and Friday, relax over the weekend, and then return on Monday and Tuesday to finish the treatment. In response to this question, Dr. Kagen said it is difficult to discern whether her improvement is due to the half-dose she received or the time and energy she saved with half the number of appointments.

What does remission mean, and how does one know if they’re in remission? What can help it occur?

The term “remission” is often used instead of the word “cured” because at this time there is no cure for myositis and no known cause for the illness. Dr. Kagen added that remission means either a resolution or an improvement in the problems a patient has experienced due to the diagnosis, such as a loss of function, loss of strength, and laboratory abnormalities. Some patients experience complete remission while others experience partial remission. However, it is unclear how to determine whether or not remission is long lasting.

Are “tired” feelings from the medications or from the myositis?

For those with steroids given by infusion, Ms. Leff mentioned that patients often report feelings of extreme weakness and exhaustion following it. Many people find themselves needing to take a nap during the day, and others hardly have the energy to continue infusion. For many people, the side effects are short lived; for others, it can last longer. Steroids like prednisone have many effects, many of which are psychological. Symptoms often include sleep difficulties or increased sensitivity. When someone doesn’t feel good, things are more difficult or frustrating; but when they’re doing better, the amount of prednisone may be diminished or withdrawn. It’s not easy to know if the symptoms of fatigue indicate that a person needs a higher dosage or a lower dosage. Figuring out how to help the patient is the art of medicine.

Is there a timeline with myositis where remission is not possible?

Dr. Kagen said that generally speaking, every study that looks at remission and timelines has indicated that the shorter the time between onset of symptoms and initiation of medical treatments, the higher the chance of successful treatment. A short time interval may be six months or less from onset of symptoms to initiation of treatment. Unfortunately, it is a difficult diagnosis to make and often takes awhile for patients to be accurately diagnosed. Other patients are misdiagnosed for a time, while some patients even avoid visiting the doctor, delaying their treatment out of fear or denial. Though the time interval may allow some people to have remission, it doesn’t mean that that is the case for each person.

When a person is in remission, does that mean their strength has come back?

The patient participant answered that she feels her strength has returned. The hill that she had so much trouble getting up is not so difficult anymore. When she began the medications, her CPK level went down and as she began to get better, she got stronger. However, she pointed out that she is not as strong as she was before myositis. She was very active and used to lift weights, run about four days a week, and regularly played tennis. As recently as two weeks ago, she began physical therapy and it is the first time she has exercised in five years. She said that after the first therapy appointment she was sore and tired, but each time she goes she finds it easier to complete the exercises.

For the patient participant, was there a turnaround in her health that led to remission?

The patient participant said that her improvement was gradual. She did not have a time that she would consider as the turnaround point. She tried a number of medications that didn’t work and was taking a mixture of different medications all at once. As the patient was weaned off the different medications, she and her husband would look at which medications were being lowered and chart the ups and downs she was experiencing. They discovered that there was really no rhyme or reason for the flares and timing of medications or type of medications. While weaning off a medication, however, the patient participant often experienced a flare. Sometimes she continued to improve, while other times she got worse. Each time she felt worse, it was devastating for her. She never gave up, though, and very gradually, she began to get better. The patient participant proudly shared that her current CPK count is well within a normal range. The audience applauded her progress.

Does remission mean a reduced CPK count or an increase in physical ability?

First, the patient participant addressed the question. She said that for her, she did concentrate on the numbers being normal. Even with a CPK count within the normal range, her physical strength is not back where it was before she was sick. She has accepted that she is not the same person as before; as a result, she cannot expect herself to return to the same abilities. She used to ride bikes with her son all the time, and now she’s on a bike in physical therapy and finding some difficulty.

Dr. Kagen mentioned that he had a number of thoughts about the patient participant sharing her story with the group. He said that her story gives us all hope that things can get better -- and they can. However, for many of us, remission will not happen. For some people, the relationship between laboratory tests and full function is not always the same. Some function well but have poor lab test results, while others are not doing well but have okay lab test results. It’s hard to make a distinction about what indicates that a person is doing better. It’s more of a feeling or “gestalt” that you get about being better. At the same time, we respect the CPK numbers and use them as important indices in treatment.

Is there anything promising in terms of stem cell research and myositis?

Dr. Kagen explained that stem cells are currently more utilized as treatment for people diagnosed with a type of dystrophy. Since there are very few patients with myositis, it can be hard to do a scientific study with such a rare disease and unknown outcome. For children with muscular dystrophy, we know the outcome and it isn’t so great. Children volunteer for experimental studies, such as using stem cell research, because it is their best hope for improvement. That is not the case with myositis. Though we do not have a cure for myositis, we do have a lot of treatments that work. Some people with myositis may say, “give me anything,” but others say they would rather wait until more is known about the experimental treatment. Therefore, it is hard to find enough people to complete a study. Dr. Kagen mentioned that most myositis studies are at the beginning stages and that a number of oral presentations have been made at meetings. The patient participant has NOT been involved in any clinical studies.

Myositis Workshop at HSS
Learn more about the Myositis Workshop, a free support and education group held monthly at Hospital for Special Surgery.